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INHERITED METABOLIC DISEASE BENEFITS Select formula, low protein food and drugs may be included as an Inherited Metabolic Disease Benefit for clients who meet the established criteria. A. Formula and Low Protein Food In addition to being an eligible Saskatchewan Health beneficiary, clients must: 1. be diagnosed with an inherited metabolic condition (may or may not be screened through newborn screening), 2. be prescribed treatment that includes formula that is used to replace an element of a typical diet (e.g. protein), 3. be prescribed formula that is only a component of their diet and is/will be less than 90% of their caloric intake after age one, 4. be prescribed formula that is not available through local suppliers; and, 5. follow a strict diet. If the diet is not followed, symptoms will be severe and include, but are not limited to, cognitive impairment and/or physical disability such as blindness. Eligible clients receive the following benefits at no charge: 1. Formula from the list of approved products.* 2. Low protein food from the list of approved products.* * The legislative authority for the formula and the low protein food is The Saskatchewan Aids to Independent Living Regulations, 1976 (Saskatchewan Regulation 292/76). 1. Formula FORMULA Abbott Nutricia (continued) Cyclinex 1 Cyclinex 2 400g 400g Glutarade Junior GA-1 Drink Mix MSUD Aid 400g 200g Glutarex-1 Glutarex-2 400g 400g MSUD Analog MSUD Maxamaid 400g 454g Hominex-1 Hominex-2 400g 400g MSUD Maxamum Periflex Advance 454g 454g I-Valex 1 I-Valex 2 400g 400g Periflex Infant Periflex Junior 400g 454g Ketonex-1 Ketonex-2 400g 400g Periflex Junior Plus Phenylade 60 400g 454g Phenex 1 Phenex 2 400g 400g Phenylade Phenylade Amino Acid Blend 454g 454g Pro-Phree Propimex 1 400g 400g Phenylade Essential Phenylade GMP 454g 400g Propimex 2 Tyrex-1 400g 400g Phenylade MTE Amino Acid Blend TYR Anamix Next 454g 400g Tyrex-2 Cambrooke 400g UCD Anamix Junior XLYS Xtrp Analog 400g 400g XLYS Xtrp Maxamaid XLYS Xtrp Maxamum 454g 454g 400g 454g Bettermilk Camino Pro PKU Mead Johnson 49g-52g 140ml BCAD-1 454g XMET Analog XMET Maxamaid BCAD-2 PFD-1 454g 454g XMET Maxamum XMTVI Analog 454g 454g PFD-2 Phenyl-Free 1 454g 454g XMTVI Maxamaid XMTVI Maxamum 454g 454g Phenyl-Free 2 Phenyl-Free 2 HP 454g 454g XPhe Maxamaid XPhe Maxamum 454g 454g Tyros-1 Tyros-2 Nutricia 454g 454g XPHE Xtyr Analog XPHE Xtyr Maxamaid 400g 454g Acerflex 454g XPHEN TYR Maxamaid XPHEN TYR Maxamum 500g 500g Complex Essential MSD Drink Mix Complex Junior MSD Drink Mix 454g 400g XPTM Analog Vitaflo 400g Complex MSUD Amino Acid Blend Duocal 454g 400g EAA Supplement HCU Cooler 12.5g 130ml Glutarade Essential Glutarade GA-1 AA Blend 454g 454g HCU Cooler 20 HCU Express 174ml 25g 2. Low Protein Food LOW PROTEIN FOOD Baking Mixes Breads Aproten Flour Mix (Farina) Cambrooke Baking Mix Cambrooke Mix Quick Cambrooke Wheat Starch Country Sunrise Muffin Mix Country Sunrise Pancake Mix Dietary Specialties Bread Machine Mix Dietary Specialties Low Protein Baking Mix Juvela Low Protein Mix Nutricia Loprofin All Purpose Baking Mix Nutricia Maddy's Homestyle Muffin Mix Nutricia Wel-Plan Wheat and Corn Starch Baking Mix Taste Connections Low Protein Bread Mix Taste Connections Low Protein Versa Mix Taste Connections Multi-Baking Mix Taste Connections Wheat Starch Rice Aproten Chicchi/Riso Rice Cambrooke Short Grain Rice Dietary Specialties Imitation Rice Nutricia Loprofin Rice Promin Pasta Cous Cous Promin Pasta Imitation Rice Meat Substitutes and Cheese Cambrooke Brookelyn Dogs Cambrooke Camburgers Cambrooke Cheese Shreds Cambrooke Cheese Singles Cambrooke EGGZ Cambrooke GO! Pockets Cambrooke Meatballs Cambrooke Mini Pockets Cambrooke Pierogi Cambrooke Artisan Bread Cambrooke Bagels Cambrooke Breakfast Bars Cambrooke Brookelyn Dog Buns Cambrooke Camburger Buns Cambrooke Cinnamon Raisin Swirl Bread Cambrooke Homestyle Sliced White Bread Cambrooke Pita Pockets Cambrooke Scones Cambrooke Southwestern Biscuits Cambrooke The Bigger Bagels Cambrooke Tortilla Wraps Cambrooke Ravioli Canbrands PaneRiso Egg Replacer Country Sunrise Burger Mix Country Sunrise Imitation Scrambled Egg and Omelette Mix Country Sunrise Peanut Butter Spread Mix Country Sunrise Vegetable Chicken Nugget Mix Country Sunrise Vegetable Hot Dog Mix Dietary Specialties Peanut Butter Flavoured Spread Ener-G Egg Replacer Walden Farms Whipped Peanut Spread Cambrooke Tuscan Pizza Crusts Cereals Dietary Specialties Porridge Nutricia Loprofin Breakfast Cereal Loops Nutricia Milupa LP-Flakes Nutricia Milupa LP-Fruity Cereal Mix Promin Pasta Meal (Porridge) Pastas Aproten Pasta, Assorted Cambrooke Pasta, Assorted Dietary Specialties Pasta, Assorted Nutricia Loprofin Pasta, Assorted Promin Pasta, Assorted Snacks Aproten Biscotto Aproten Cracker Toast/Fette Tostate Aproten Crispbread/Rusks Nutricia Loprofin Crackers Miscellaneous Cambrooke Alfredo Sauce Mix Cambrooke Chicken Consomme and Seasoning Cambrooke Shake N'Cheese Country Sunrise Cheese Flavoured Sauce Mix Country Sunrise Instant Mashed Potato Mix Dietary Specialties Cheese Flavoured Sauce Mix Dietary Specialties Tomato Flavoured Sauce Mix La Tiara Taco Shells Nutricia Milupa LP-Drink Mix Washington Golden Seasoning and Broth Washington Rich Brown Seasoning and Broth B. Drug Benefits Please note, physicians may make individual case by case requests for medications to treat inherited metabolic diseases. Requesting physicians are asked to provide the Drug Plan with clinical information to support the request. 1. saproterin dihydrochloride (Kuvan-BPC) - Criteria For Coverage For more information on how to obtain coverage, or if you have questions regarding the criteria or billing procedures, please contact the Drug Plan. Initial Inclusion Criteria: Non-Pregnant Patients and Patients actively planning pregnancy: For the management of patients with the diagnosis of Phenylketonuria (PKU) who meet ALL of the following criteria: 1. 2. Compliance with low protein diet and formulas. Baseline blood phenylalanine (Phe) levels > 360 μmol/L despite compliance with low protein diet (require at least 2 levels during 3 to 6 month time frame). 3. Baseline protein intake assessment by a dietitian. 4. Ability to comply with medication regimen. 5. Managed by a physician specialized in metabolic/biochemical diseases. Pregnant Patients: For the management of patients with the diagnosis of Phenylketonuria (PKU) who meet ALL of the following criteria: 1. 2. Managed by a physician specialized in metabolic/biochemical diseases. Baseline blood phenylalanine (Phe) levels > 360 μmol/L despite compliance with all recommendations for dietary intervention and monitoring. Exclusion Criteria: • Known hypersensitivity to sapropterin or its excipients. • Any other contraindications. • Baseline Phe Levels <360 μmol/L for non-pregnant patients. • Baseline Phe Levels <360 μmol/L for pregnant patients. • Women who are nursing/breast feeding. • Patients not on diet or not compliant with diet. Caution: • Taking medication known to inhibit folate synthesis (e.g., methotrexate). • Any condition requiring treatment with levodopa or any PDE-5 inhibitor (e.g., sildenafil). Test for Eligibility: 72 hour Kuvan Challenge 72 hour challenge with Kuvan at 20 mg/kg/day - blood Phe concentrations are measured at the following timepoints (in hours): T -48 to T -24, T0, T4 to T12, T 24, T 48 to T 72, OR As per clinic’s protocol. Dose of Kuvan to establish clinical benefit is 20 mg/kg/day. Definition of Responder to Kuvan Challenge: For Non-Pregnant Patients and Patients actively planning pregnancy: Criteria to determine if patients have “responded” to 72 hour Kuvan challenge: Reduction in Phe blood level of at least 30% compared to baseline; AND Baseline assessment of neurobehavioural or neurocognitive impairment* and quality of life assessment due to PKU after 72 hr Kuvan challenge but before start of Kuvan therapy (this assessment does not apply to pregnant women). Note: Baseline Phe tolerance level must be documented as well as Phe tolerance levels documented at months 1-2 and 4-6 while on Kuvan therapy. Approval Duration: 6 months (KUVAN THERAPY TO BE FUNDED BY THE MANUFACTURER FOR 6 MONTHS) * For children less than 4 years of age, clinically validated age-appropriate neurobehavioural, neurocognitive, or developmental tests may be selected at the clinician’s discretion rather than PKU specific tests. Pregnant Patients: Criteria to determine if patients have “responded” to 72 hour Kuvan challenge: 1. Reduction in Phe blood level of at least 30% compared to baseline after 72 hours. For patients who meet “responder” definition to Kuvan Challenge, may be eligible for Kuvan funding if the following criteria are met: A decrease in Phe concentration to less than 360 μmol/L to be maintained for the duration of pregnancy to be eligible for continued funding. Approval Duration: Until end of pregnancy, no renewals (KUVAN THERAPY TO BE FUNDED BY THE MANUFACTURER FOR 6 MONTHS) INITIAL REQUESTS FOR FUNDING AND RENEWAL REQUESTS: (AFTER INITIAL SIX-MONTH TRIAL DEMONSTRATING RESPONSE TO KUVAN THERAPY) Non-Pregnant Patients and Patients actively planning pregnancy: For the management of patients with the diagnosis of Phenylketonuria (PKU) who meet ALL of the following criteria: 1. Compliance with low protein diet, formulas, and Kuvan; AND 2. Achieve: a) normal sustained Blood Phe levels [ > 120 μmol/L and < 360 μmol/L] (At least 2 levels measured at least 1 month apart); OR b) sustained blood Phe reduction of at least 30% (At least 2 levels measured at least 1 month apart) compared to baseline if the Phe baseline level is < 1200 μmol/L; OR c) sustained blood Phe reduction of at least 50% (At least 2 levels measured at least 1 month apart) compared to baseline if the Phe baseline level is > 1200 μmol/L; AND 3. Demonstrated increase of dietary protein tolerance based on target set between the clinician and patient; AND 4. Clinically meaningful age-appropriate improvement in: Neurobehavioural or neurocognitive function or impairment for patients with such impairments as determined by peer reviewed clinically validated scales; OR Demonstrated improvement in Quality of Life using peer reviewed validated scales; AND 5. Managed by a physician specialized in metabolic/biochemical diseases. Dosage: Up to a maximum of 20 mg/kg per day. Approval Duration: 1 year Pregnant Patients: No renewals will be considered. Note: Patients enrolled in sapropterin clinical trials PKU 015, PKU 016 and Kuvan Assistance Program would have to meet provincial coverage criteria in order to quality for funding. 1. Coverage for Fabry Disease Saskatchewan participates in the Canadian Fabry Disease Initiative (CFDI). Prescribers, pharmacists or patients with questions regarding coverage of enzyme replacement therapy for the treatment and management of Fabry Disease are asked to call the Drug Plan and Extended Benefits Branch for more information.